Unilateral megalencephaly associated with neonatal high output cardiac failure

Academic Article


  • High-flow cerebral arteriovenous fistulae are well known to present in the neonatal period with macrocephaly, cranial bruits and high-output cardiac failure. This report describes a newborn infant with such a clinical presentation, who had unilateral megalencephaly without macroscopic arteriovenous shunts. Ultrasound Doppler examination of the carotid and cerebral vessels showed diastolic flow, or a decreased pulsatility index, consistent with decreased intracranial vascular resistance. The ipsilateral cerebral arteries and veins were markedly enlarged at angiography and at post-mortem examination, but there was no arteriovenous malformation. The infant expired from high-output cardiac failure and hypoglycemia. It is postulated that the high-output cardiac failure was due to increased blood flow through the enlarged, dysplastic cerebral hemisphere. © 1990 Springer-Verlag.
  • Authors

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    Digital Object Identifier (doi)

    Author List

  • Walters BC; Burrows PE; Musewe N; Chuang SH; Armstrong D
  • Start Page

  • 123
  • End Page

  • 125
  • Volume

  • 6
  • Issue

  • 3